Risk factors for development of desmoid tumours in familial adenomatous polyposis.

Abstract

Desmoid tumours (DTs) are the primary cause of death of patients with familial adenomatous polyposis (FAP) following restorative proctocolectomy. The aim of this study was to identify risk factors for DT in a French population. Clinical data for 442 patients with FAP from 1983 to 2004 were reviewed retrospectively. A total of 124 DTs were documented in 50 patients (25 female). DT sites were mesenteric (73 tumours), abdominal wall (44) and extra-abdominal (seven). Female patients developed DT earlier than males. Although DTs appeared after colectomy in 34 patients, the type of surgery did not influence the risk of DT. An identified point mutation in the adenomatous polyposis coli (APC) gene after codon 1444 was a significant risk factor (hazard ratio 3.3 (95 per cent confidence interval 1.5 to 7.3)). Belonging to a family affected by DT did not increase the individual's risk in this population. No risk factor for life-threatening mesenteric DT could meaningfully modify the management of patients with FAP.